Pulmonology Book

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Interstitial Lung Disease

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  1. Definition
    1. Chronic, nonmalignant, noninfectious diseases
    2. Lower respiratory tract disease
      1. Distal lung parenchyma disease
      2. Alveolar wall inflammation and derangement
  2. Causes (Mnemonic: DISCO)
    1. Drugs (See below)
    2. Idiopathic Pulmonary Fibrosis
    3. Sarcoidosis
    4. Collagen Vascular Disease
    5. Occupational Exposure
      1. Asbestos
      2. Fumes and gasses
  3. Causes: Non-Granulomatous Causes
    1. Occupational Exposure
    2. Medications
      1. Antibiotics (e.g. Nitrofurantoin)
      2. Chemotherapeutics (e.g. Methotrexate)
    3. Radiation
    4. Aspiration Pneumonia
    5. Post ARDS changes
    6. Idiopathic Pulmonary Fibrosis
    7. Collagen Vascular Disease
    8. Goodpasture's Syndrome
    9. Eosinophilic Pneumonia
    10. Amyloidosis
    11. Graft versus Host disease (Bone Marrow Transplant)
  4. Causes: Granulomatous Causes
    1. Hypersensitivity Pneumonitis (Organic dust)
    2. Inorganic dust (Beryllium silica)
    3. Sarcoidosis
    4. Langerhans Cell Granulomatosis
    5. Granulomatous Vasculitis
      1. Wegener's Granulomatosis
      2. Allergic Granulomatosis of Churg-Strauss
      3. Lymphomatoid granulomatosis
    6. Bronchocentric granulomatosis
  5. Demographic Breakdown
    1. Age 20 to 40 years
      1. Sarcoidosis
      2. Collagen Vascular Disease
      3. Eosinophilic Granuloma or Pulmonary Histiocytosis X
      4. Lymphangioleiomyomatosis
    2. Age over 40 years
      1. Idiopathic Pulmonary Fibrosis
    3. Female Predominance
      1. Collagen Vascular Disease
      2. Lymphangioleiomyomatosis
      3. Tuberous Sclerosis
    4. Male Predominance
      1. Pneumoconiosis (Asbestosis)
    5. Tobacco exposure
      1. More likely with Tobacco exposure
        1. Eosinophilic Granuloma or Pulmonary Histiocytosis X
        2. Respiratory Bronchiolitis (Smoker's Bronchiolitis)
        3. Diffuse alveolar hemorrhage: Goodpasture's Syndrome
      2. Less likely if Tobacco exposure
        1. Idiopathic Pulmonary Fibrosis
        2. Sarcoidosis
        3. Hypersensitivity Pneumonitis
  6. Symptoms
    1. Historical data
      1. Complete occupational and environmental exposures
      2. Complete medication history
    2. Exertional Dyspnea
    3. Chronic Dyspnea
    4. Fatigue
    5. Malaise
  7. Signs
    1. Inspiratory crackles (rales) at posterior lung bases
    2. Pulmonary Hypertension
    3. Fingernail Clubbing
      1. Suggests Idiopathic Pulmonary Fibrosis
      2. May suggest bronchogenic carcinoma
  8. Labs
    1. ACE Level is non-specific and not useful
    2. Erythrocyte Sedimentation Rate (ESR)
      1. Increased
    3. Complete Blood Count (CBC)
      1. Polycythemia (rare)
    4. Arterial Blood Gas (ABG)
      1. Hypoxemia
  9. Diagnosis
    1. Pulmonary Function Tests
      1. Restrictive pattern
      2. Reduced Total Lung Capacity
      3. DLCO decreased
    2. Bronchoalveolar Lavage
      1. Alveolar cells reflect type of inflammation
  10. Radiology
    1. Chest XRay
      1. Lung parenchymal change (90%)
      2. Increased interstitial markings
      3. Non-resolving infiltrates
    2. Gallium Lung scanning
      1. Diffuse inflammation

Lung Diseases, Interstitial (C0206062)

Definition (MSH)A heterogeneous group of noninfectious, nonmalignant disorders of the lower respiratory tract, affecting primarily the alveolar wall structures but also often involving the small airways and blood vessels of the lung parenchyma. "Interstitial" refers to the fact that the interstitium of the alveolar walls is thickened, usually by fibrosis. This group of diseases is usually inflammatory. (Dorland, 27th ed; Wyngarden, Cecil Textbook of Medicine, 19th ed, p396)
Definition (CSP)heterogeneous group of noninfectious, nonmalignant disorders of the lower respiratory tract, affecting primarily the alveolar wall structures but also often involving the small airways and blood vessels of the lung parenchyma; may lead to respiratory insufficiency and death.
ConceptsDisease or Syndrome (T047)
EnglishILD, ILD - Interstitial lung disease, INTERSTITIAL LUNG DIS, interstitial lung disease, Interstitial Lung Diseases, LUNG DIS INTERSTITIAL
Spanishenfermedad del intersticio pulmonar, enfermedad pulmonar intersticial
Parent ConceptsLung diseases (C0024115), Connective Tissue Diseases (C0009782)
SourcesCOSTAR, CSP, CST, MSH, NDFRT, OMIM, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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